Degos' disease (also known as Kohlmeier disease and malignant atrophic papulosis (MAP)) is a rare vasculopathy (around 200 reported cases) characterized by thrombosis in small to large vessels. See, e.g., Lester and Rapini (2009) Curr Opin Gastroenterol 25:66-73 and Englert et al. (1984) Br Med J 289:576. Although generally considered to be of unknown etiology, Degos' disease has been associated with viral infections (e.g., B19 parvovirus and HIV) and autoimmune disorders such as lupus erythematosis (LE), dermatomyositis, and primary antiphospholipid syndrome (APS). See, e.g., Crowson et al. (2002) J Cutan Pathol 29:596-601; Englert et al. (1984), supra; Heymann (2009) J Am Acad Dermatol 61:505-506; Durie et al. (1969) Arch Dermatol 100(5):575-581; Tsao et al. (1997) J Am Acad Dermatol 36:317-319; and Requena et al. (1998) J Am Acad Dermatol 38:852-856. Some forms of Degos' disease may be familial. See, e.g., Katz et al. (1997) J Am Acad Dermatol 37:480-484 and Penault et al. (2004) Ann Dermatol Venereol 131:989-993. Degos can occur in patients of any age, yet it appears to preferentially affect men over women at a ratio of approximately 3 to 1. See, e.g., Katz et al. (1997), supra; Torrelo et al. (2002) Br J Dermatol 146:916-918; and Wilson et al. (2007) Pediatr Dermatol 24(1):18-24.
Degos can manifest as a benign, purely cutaneous form or as an aggressive, multiorgan, systemic form, the latter of which is generally fatal within one to twelve years after diagnosis. Scheinfeld (2007) Clin Exp Derm 32:483-487. The phenotypic hallmark of cutaneous Degos' disease is the appearance of one or more erythematous, reddish-colored papules on the skin, which papules scar over with white, atrophic centers.
Death occurs in nearly all patients with the systemic form of Degos' disease, the patients having an average life expectancy after systemic involvement of around two to three years. See, e.g., Scheinfeld (2007), supra. Patients usually die from intestinal perforation with or without septic complications; however, death may alternatively result from intestinal infarction, cardiopulmonary collapse, and/or neurological infarction and hemorrhage. Id. See also High et al. (2004) J Am Acad Dermatol 50(6):895-899.
A standard medical treatment for Degos' disease has not been defined. Many therapeutic agents have had only marginal and/or inconsistent success in treating the disease. See, e.g., Scheinfeld (2007), supra. For example, some Degos patients benefited from intravenous immunoglobulin therapy, but at present there appears to be no way of predicting which patients would respond to such therapy, see, e.g., Dyrsen et al. (2008) J Cutan Pathol 35 (Suppl 1):20-25; Zhu et al. (2007) Br J Dermatol 157(1):206-207; and De Breucker et al. (2008) Acta Clin Belg 63(2):99-102 (Abstract).
In view of the foregoing, it is clear that there is a need for new approaches and better methods to treat patients with Degos' disease.